Serum angiotensin-converting enzyme has low diagnostic value for pediatric sarcoid uveitis.

PURPOSE: Sarcoid uveitis is rare in the pediatric population. Early diagnosis is challenging and is crucial, due to more severe complications. Diagnosis relies on various criteria, including elevated angiotensin-converting enzyme (ACE) levels. The objective of this study was to evaluate the diagnostic value of serum ACE levels in the diagnosis of pediatric sarcoid uveitis. METHODS: This was an observational retrospective multicenter study of chronic, severe pediatric uveitis between 2013 and 2019 in two French tertiary referral centers. RESULTS: An ACE assay result was available for 105 patients. Nine patients were diagnosed with sarcoid uveitis. The diagnostic values were as follows: sensitivity=22.2%, specificity=87.5%, positive predictive value=14.3%, negative predictive value=92.3%, positive likelihood ratio=1.8, and negative likelihood ratio=0.9. CONCLUSION: The diagnostic performance of ACE in pediatric sarcoid uveitis was found to be poor. NPV exceeded 90% but was based on a significant number of false negatives, indicating a high risk of misdiagnosis. Likelihood ratios confirmed the limited diagnostic value of ACE. Considering age groups and clinical manifestations may enhance precision but requires larger studies. Serum ACE used as a diagnostic marker in pediatric sarcoid uveitis warrants caution and should be interpreted alongside other factors.

[Immunosuppressive therapy in severe or chronic pediatric uveitis: Review of the literature]. / Les immunosuppresseurs dans les uvéites pédiatriques sévères ou chroniques : revue de la littérature.

Immunosuppressants are prescribed for pediatric uveitis in cases of severe involvement affecting the prognosis for vision or life, in cases of recurrent or chronic uveitis to achieve corticosteroid sparing, or in cases of corticosteroid resistance. Immunosuppressants used in children include antimetabolites (methotrexate, mycophenolate mofetil, azathioprine), cyclosporine, tacrolimus, and biologics, including infliximab, adalimumab, anakinra, canakinumab, and tocilizumab. The mechanisms of action and indications of the various immunosuppressants are described in this review.

[Juvenile dermatomyositis and new autoantibodies: Cases and review]. / Dermatomyosite juvénile et nouveaux auto-anticorps : à propos de quatre observations.

Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in children. Its diagnosis is usually made on a clinical basis following the criteria of Bohan and Peter (1975). Recently, the presence of myositis-specific autoantibodies (MSAs) have started to be associated with specific outcome in adult patients; the diagnosis and prognosis value of these autoantibodies remains to be identified in children. We report four cases of JDM with MSAs focusing on clinical, biological, and radiological manifestations, and then we describe associated treatment. The cohort comprises four girls with an average age of 8.5 years. The time to diagnosis was 1 week to 4 months. For these patients, the immunologic study found one patient positive for the MDA5 antibody (or CADM 140), one positive for the TIF1γ antibody (or p155/140), and two patients positive for the NXP2 antibody (or p140/MJ). Each patient showed specific and characteristic cutaneous manifestations. For example, the girl positive for the TIF1γ antibody presented the most severe skin disease with urticaria, face edema, and vascularity of the neck and shoulders. However, regarding muscular features, proximal weakness was present in most of the cohort, except for the child positive for the MDA5 antibody, who presented no sign of muscular disease at the beginning with low CK levels. Importantly, acute pancreatitis also affected this patient. Concerning radiological indications, muscular MRI evidenced hyperinflammation, a sign of diffuse myositis, in all these patients. Treatments consisted in corticosteroids together with methotrexate or mycofenolate mofetil associated or not with intravenous immunoglobulin therapy. This report highlights the importance of systematic detection and analysis of MSA in diagnosis and characterization of JDM, and describes a new approach that would allow more focused treatments and be a useful predictor of clinical complications and prognosis in JDM-affected subjects.

[Nicolau syndrome after intramuscular injection]. / Complication d'une injection intramusculaire ou syndrome de Nicolau.

Nicolau syndrome is a rare, potentially severe complication that may occur after any drug injection, particularly after intramuscular injection. It is characterized by the acute onset of cutaneous and soft-tissue aseptic necrosis. Here, we report the case of a 14-year-old boy diagnosed with Nicolau syndrome on the right lower limb, after a benzathine-penicillin intramuscular injection for suspected rheumatic fever. The short-term progression was marked by uncomplicated rhabdomyolysis and the constitution of homolateral testicular torsion. The cutaneous-muscular disorders evolved favorably under symptomatic treatment. We discuss this insufficiently known complication of intramuscular injection, which may motivate reduced use of this route of drug administration in children and strict adherence to the procedure. Furthermore, it is important to note that Nicolau syndrome may evolve to homolateral testicular torsion, as, to the best of our knowledge, is reported for the first time in this case.

[Values of clinical signs and rapid diagnostic test in the diagnosis of influenza A (H1N1) new variant in pediatric emergency department]. / Valeurs des signes cliniques et d'un test de diagnostic rapide dans le diagnostic de la grippe A (H1N1) nouveau variant aux urgences pédiatriques.

AIM OF THE STUDY: To evaluate the values of clinical signs and the rapid diagnostic test (RDT) in the diagnosis of influenza H1N1 new variant in the pediatric emergency room. METHOD: From 18 August to 1st December 2009, children admitted to the pediatric emergency department of CHU Nord (Marseille, France) and suspected of flu according to French guidelines, were tested for influenza using both an influenza RDT and a polymerase chain reaction (PCR) assay specific for H1N1. From 3 November to 3 December, clinical signs were also noted (fever, headaches, myalgia-arthralgia, shivers, diarrhea). RESULTS: A total of 1122 children were tested: 367 children (32.1%) had a positive specific PCR. The RDT value was: sensitivity 65.2% [55.8-73.6], specificity 99.5% [98.1-100], positive predictive value (PPV) 97.5% [91.8-100], negative predictive value (NPV) 91.2% [90.3-91.5], positive likelihood ratio (LRP) 153.7 [53.5-452.9] and negative likelihood ratio (LRN) 0.393 [0.387-0.411]. Clinical data were available for 504 children (328 over 2 years of age). In children more than 2 years of age and in multivariate analysis, headaches were the only sign significantly associated with a positive PCR (aOR=2.53 [1.25-5.12]). Overall, headaches and/or myalgia-arthralgia were valuable indicators for clinical diagnosis of flu, with a 75.8% NPV. Among children with a positive PCR, diarrhea was more frequent in children under 2 years of age (OR=2.76 [1.19-6.40]). The sensitivity of the RDT improved (90.9% [85.2-94.6]) when flu signs were also present. CONCLUSION: Associating clinical signs and RDT for the diagnosis of influenza A (H1N1) new variant in a pediatric emergency room improves selection of children requiring appropriate antiviral treatment.

[Pediatric Mediterranean spotted fever]. / La fièvre boutonneuse méditerranéenne chez l'enfant.

In France, Mediterranean spotted fever due to Rickettsia conorii is mainly observed in the South-east. Rhipicephalus sanguineus, the brown dog tick, is the vector and the reservoir of the disease. Clinical diagnosis relies on the association of fever, rash and inoculation's scar during summertime. Pathological lesion is a diffuse vasculitis. Severity is often explained by a delay in diagnosis, but prognosis is good in pediatrics. Treatment relies on doxycycline and on macrolids in children under eight.